Adult-onset leukodystrophy with axonal spheroids

This code identifies a rare, progressive neurological disorder affecting adults, characterized by the degeneration of white matter in the brain and spinal cord, specifically with the presence of axonal spheroids (swollen axons). It leads to a range of neurological symptoms including cognitive decline, motor dysfunction, and psychiatric disturbances, typically manifesting in adulthood.

Apply this code when documentation confirms a diagnosis of adult-onset leukodystrophy with the specific pathological finding of axonal spheroids. This diagnosis is often established through genetic testing, neuroimaging (MRI showing white matter changes), and sometimes brain biopsy confirming the presence of spheroids.

• Ensure the documentation explicitly states "adult-onset" and mentions "axonal spheroids" to support this specific code.
• Distinguish from other leukodystrophies that do not involve axonal spheroids or have a pediatric onset.
• Sequence this code as a primary diagnosis when it is the reason for the encounter, or as a secondary diagnosis if it is a co-morbidity affecting treatment.